4-17-2021. Starting meds

She is starting her medications: immunosuppressant for at least a year, and corticosteroid for a half year, to hopefully get her immune system to chill and “reset” itself, stopping the complement cascade problem(s). She is also taking antibiotics on the weekends to give her some added protection.

The steroid’ll raise her blood pressure, which can strain her kidneys. So we are now monitoring her blood pressure and she is on a low sodium diet. She also needs to take extra Calcium and Vitamin D. Some medications might make her prone to skin cancer, so we need to be diligent with sunscreen. Good thing she likes ice skating!

We do not need to quarantine, THANK GOD! But we do need to avoid sick people. To answer the common question, “How is she doing?” She’s begrudgingly yet obediently taking her medicine, occasionally wondering, “Why me?” Then she runs off to play.

This weekend we go to Cincinnati Children’s Hospital for a 2nd opinion. Honestly we don’t expect any different advice, because there’s not much difference in course of therapy for such a rare condition. We are mostly deciding who we want to do continuity of care with. It’s difficult for our current nephrologist because this is so rare that she’s probably never seen this before. But she’s been working hard for Alyssa, which we are thankful for. We just need to do our due diligence and get that 2nd opinion. We chose Cincinnati because it is world renown in pediatric rare kidney diseases. We also considered Iowa, and that is also still in the back of our minds.

In terms of what to expect, she will be on the immunosuppression therapy at least a year. If this doesn’t work, then it’s a matter of time before her kidneys worsen, and a long journey of medications and low sodium, low potassium, low phosphorous diet, blood pressure meds, complement inhibitors, and possibly experimental medications to postpone end stage renal disease, where she would need dialysis or kidney transplant. And even if she gets a kidney transplant, her immune system may continue to attack the new kidney. And then she may need another transplant. And another. So we hope that this immunosuppression works, and that she will go into remission, ideally for the rest of her life. Some people go into remission, then later get sick, and it reactivates the whole problem all over again and they need to restart immunosuppression.

How are the rest of us doing? Better. We were grieving her diagnosis, but are slowly coming to terms. For Mom, it’s been painful to be so helpless. I found some solace in knowing we have the same blood type, that maybe someday if needed, I can give her a kidney and buy her more dialysis-free years. For Dad, it’s been a spiritual struggle as God calls him to lay it all down before Him and earnestly pray. For Brother, he’s not really understanding, but trying to be supportive.

On the bright side, Alyssa is C3 nephritic factor and factor H antibody positive. Apparently those kids often do better, not sure if it’s because they’re aggressively treated or if it’s a milder phenotype. Either way, we rejoice in these encouragements! Hers was also diagnosed before there was any tubular scarring, so that is another good sign. This is why she feels fine, because there’s no damage to kidneys, and we just happened to catch her odd numbers. So we are thankful and cherish each day.